Renal tumors
یفطل دازهب رتکد
History
• First nephrectomy: 1861
• Harris: 1882 : 100 nephrectomies
• Grawitz: 1883: Adrenal rest origin corrected by: Albarran & Imbert
• Radical nephrectomy: 1960s
Classification
• Malignant
• Benign
Classification
Malignant
:
• RENAL CELL CARCINOMA (RCC)
• Clear cell RCC
• Multilocular cystic clear cell renal cell neoplasm of low malignant potential
• Papillary RCC
• Chromophobe RCC
• Hybrid oncocytic chromophobe tumor
• Carcinoma of the collecting ducts of Bellini
• Renal medullary carcinoma
• MiT family translocation RCC
• Mucinous tubular and spindle cell carcinoma
• Tubulocystic RCC
• Acquired cystic disease–associated RCC
• Clear cell (tubulo) papillary RCC
Classification
• UROTHELIUM-BASED CANCERS
• Urothelial carcinoma
• Squamous cell carcinoma
• Adenocarcinoma
• SARCOMAS
• Leiomyosarcoma
• Liposarcoma
• Other sarcomas
• NEPHROBLASTIC TUMORSa
• Nephrogenic rests
• Nephroblastoma (Wilms tumor)
Classification
• CARCINOMA ASSOCIATED WITH NEUROBLASTOMA Neuroendocrine Tumors • Carcinoid (low-grade neuroendocrine tumor)
• Neuroendocrine carcinoma (high-grade neuroendocrine tumor) • Primitive neuroectodermal tumor
• Neuroblastoma
• Pheochromocytoma
• Hematopoietic and Lymphoid Tumors • Lymphoma
• Leukemia
• Plasmacytoma
Germ Cell Tumors
• Teratoma
• Choriocarcinoma • METASTASIS
Classification
Benign
• CYSTIC LESIONS • Simple cyst • Hemorrhagic cyst • SOLID LESIONS • Angiomyolipoma • Oncocytoma• Papillary adenoma (renal adenoma)
• Metanephric tumors (adenoma, adenofibroma, stromal tumor) • Congenital mesoblastic nephroma
• Cystic nephroma/mixed epithelial stromal tumor • Reninoma (juxtaglomerular cell tumor)
• Renomedullary interstitial tumor • Leiomyoma
• Fibroma
• Hemangioma • Lymphangioma • Schwannoma
Classification
Benign
• VASCULAR LESIONS
• Renal artery aneurysm
• Arteriovenous malformation • PSEUDOTUMOR • INFLAMMATORY • Abscess • Focal pyelonephritis • Xanthogranulomatous pyelonephritis • Infected renal cyst
• Tuberculosis
Imaging and Clinical Risk Stratifcation of Renal Masses
Models and nomograms
• Predict the likelihood of malignancy • Generally in the range 0.55 to 0.65
The strongest predictors of malignancy
• male sex
Imaging and Clinical Risk Stratifcation of Renal Masses
• Sex:
• Men: threefold increased risk of malignancy compared with women. • Although benign histology is more common in women, RCC still
Imaging and Clinical Risk Stratifcation of Renal Masses
• Size:
• The likelihood of malignancy increases (1.3 per cm) of tumor diameter. • Tumors smaller than 1 cm: 46% benign - 2% high-grade RCC
Tumors larger than 7 cm: 6% benign - 58% high-grade RCC
RCC
• Incidence
• 2-3% of all adult malignancies
• The most lethal of the common urologic cancers
• 16 new cases per 100,000 population per year, with a male-to-female predominance of 1.9 to 1
• Disease of older adults (55 and 75 years of age)
• Diagnosis of renal cancer has increased more rapidly in <40 years of age than any other age group
Imaging and Clinical Risk Stratifcation of Renal Masses
• The incidence of RCC has increased since the 1970s by an
average of 3% per year (use of ultrasonography and CT)
• incidentally discovered: ↑
• localized tumors: ↑
• 5-year survival: improved for patients with this stage of disease
Imaging and Clinical Risk Stratifcation of Renal Masses
• Childhood renal tumors: 7% of all pediatric cancers
• RCC is relatively uncommon in this population
• 90% of renal tumors in children: Wilms tumors or nephroblastomas
• RCC representing only 1% to 6%
• RCC = Wilms tumor during the second decade of life.
• Mean age for RCC in children is 8 to 9 years, boys = girls.
Etiology
• Tobacco exposure
• Relative risks: 1.4 to 2.5
• All forms of tobacco use
• Risk increases with cumulative dose or pack-years
• Relative risk is directly related to duration of smoking
• begins to fall after cessation
Etiology
Obesity
• Increased relative risk of 1.07 for each additional unit of
body mass index
• RCC is more prevalent in patients with higher BMI
• Increased BMI is associated with lower stage RCC at presentation, a
so-called “obesity paradox”
• Potential mechanisms:
• increased insulin-like growth factor-1 expression • increased circulating estrogen levels
Etiology
• Hypertension
• Third major causative factor for RCC
• Diuretics and other antihypertensive medications have
also been implicated, particularly for papillary RCC
• It is the underlying disorder, hypertension, rather than the
treatment that increases the risk of RCC
• The proposed mechanisms:
• hypertension-induced renal injury
Etiology
• In animal models
• Viruses
• lead compounds
• more than 100 chemicals such as aromatic hydrocarbons
No specific agent has been definitively established as causative in human
RCC
Increased relative risks for RCC:
workers in the metal, chemical, rubber, and printing industries
those exposed to asbestos or cadmium
Etiology
• RCC is more common among individuals with low socioeconomic status
and urban background
• The typical modern Western diet, increased intake of dairy products,
and increased consumption of coffee or tea have been associated
with RCC
Etiology
• Regular usage of NSAIDs was associated with a relative risk of 1.51
• aspirin and acetaminophen were not associated with increased risk
• Retroperitoneal radiation therapy: appears to be a risk factor for RCC,
although the relative risks are low
Familial Renal Cell Carcinoma and Molecular Genetics
• von Hippel-Lindau Disease, VHL Gene, and Genetics of
Clear Cell RCC
• RCC
• Pheochromocytoma
• retinal angiomas
• hemangioblastomas of the brainstem, cerebellum, or spinal cord
• RCC develops in about 50% of patients with VHL disease
• early age at onset (often in the third to fifth decades of life)
• Bilateral
Familial Renal Cell Carcinoma and Molecular Genetics
Hereditary Papillary Renal Carcinoma Syndrome
Autosomal dominantMedian age at diagnosis: 45 years
Most patients developed multifocal and bilateral papillary RCC Type 1 papillary RCC
Typically do not develop tumors in other organ systems
Familial Renal Cell Carcinoma and Molecular Genetics
Hereditary Leiomyomatosis and Renal Cell Carcinoma
cutaneous and uterine leiomyomas and type 2 papillary RCC
Mean age at diagnosis: early 40s.
Often solitary and unilateral, more aggressive than other forms of familial
RCC.
Mutation: fumarate hydratase (FH) gene
Familial Renal Cell Carcinoma and Molecular Genetics
Succinate Dehydrogenase Renal Cell Carcinoma
Shares features with HLRCC
Affected individuals present with bilateral, multifocal, early onset (<40
years) renal tumors
often along with pheochromocytomas and head and neck paragangliomas.
Good prognosis for a subset of patients with SDH-RCC, particularly when
low-grade features predominate.
Familial Renal Cell Carcinoma and Molecular Genetics
Birt-Hogg-Dube Syndrome
Birt-Hogg-Dube (BHD) syndrome:
cutaneous fibrofolliculomas
lung cysts
spontaneous pneumothoraces
renal tumors primarily derived from the distal nephron
The renal tumors typically include chromophobe RCC, oncocytomas,
and hybrid oncocytic tumors
Familial Renal Cell Carcinoma and Molecular Genetics
Average : 50 years
Most renal tumors in BHD syndrome have limited biologic
aggressiveness
Familial Renal Cell Carcinoma and Molecular Genetics
Cowden Syndrome
mutations of PTEN tumor suppressor gene Termed PTEN hamartoma tumor syndrome 50% lifetime risk of female breast cancer 34% lifetime risk of RCC
10% lifetime risk of epithelial thyroid carcinoma Greater than 31-fold increased risk of RCC
should be screened with CT or MRI for RCC
Familial Renal Cell Carcinoma and Molecular Genetics
Tuberous Sclerosis Complex
autosomal dominant disorder include AML
cortical tubers
subependymal nodules or subependymal giant cell astrocytomas (SEGA) pulmonary lymphangioleiomyomatosis (LAM)
cardiac rhabdomyomas facial angiofbromas
Renal AML are typically multifocal, bilateral, and often large, occasionally requiring
angioembolization or partial nephrectomy (PN) to treat or prevent bleeding from larger (>5 cm) tumors.
